共 21 条
Low rate of cardiac events in first-degree relatives of diagnosis-negative young sudden unexplained death syndrome victims during follow-up
被引:28
作者:
van der Werf, Christian
[1
]
Stiekema, Lotte
[1
]
Tan, Hanno L.
[1
]
Hofman, Nynke
[2
]
Alders, Marielle
[2
]
van der Wal, Allard C.
[3
]
van Langen, Irene M.
[4
]
Wilde, Arthur A. M.
[1
,5
]
机构:
[1] Univ Amsterdam, Acad Med Ctr, Dept Cardiol, Ctr Heart, NL-1105 AZ Amsterdam, Netherlands
[2] Univ Amsterdam, Acad Med Ctr, Dept Clin Genet, NL-1105 AZ Amsterdam, Netherlands
[3] Univ Amsterdam, Acad Med Ctr, Dept Pathol, NL-1105 AZ Amsterdam, Netherlands
[4] Univ Groningen, Univ Med Ctr Groningen, Dept Genet, Groningen, Netherlands
[5] King Abdulaziz Univ, Princess Al Jawhara Albrahim Ctr Excellence Res H, Jeddah 21413, Saudi Arabia
关键词:
Sudden unexplained death syndrome;
Sudden cardiac death;
Arrhythmia;
Genetics;
CARDIOVASCULAR-DISEASE;
ARREST;
ADULTS;
RISK;
D O I:
10.1016/j.hrthm.2014.05.028
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
BACKGROUND Sudden unexplained death syndrome (SUDS) in young individuals often results from inherited cardiac disease. Accordingly, comprehensive examination in surviving first-degree relatives unmasks such disease in approximately 35% of the families. It is unknown whether individuals from diagnosisnegative families are at risk of developing manifest disease or cardiac events during follow-up. OBJECTIVE This study aimed to study the prognosis of first-degree relatives of young SUDS victims, in whom the initial cardiologic and genetic examination did not lead to a diagnosis. METHODS We retrieved vital status of surviving first-degree relatives from 83 diagnosis-negative families who presented to our cardiogenetics department between 1996 and 2009 because of SUDS in > 1 relatives aged 1-50 years. Moreover, we contacted relatives who previously visited our center for detailed information. RESULTS We obtained detailed information (median follow-up 6.6 years; interquartile range 4.7-9.6 years) in 340 of 417 first-degree relatives (81.5%) from 77 of 83 families (92.8%). Vital status, available in 405 relatives (97.1%), showed that 20 relatives (4.9%) died during follow-up, including 1 natural death before the age of 50. This girl belonged to a family with multiple cases of idiopathic ventricular fibrillation and SUDS, including another successfully resuscitated sibling during follow-up. Two hundred thirty-four of 340 first-degree relatives (68.8%) underwent cardiologic examination. Of these, 76 (32.5 /0) were reevaluated. Inherited cardiac disease was diagnosed in 3 families (3.6%). CONCLUSION In first-degree relatives of young SUDS victims with no manifest abnormalities during the initial examination, the risk of developing manifest inherited cardiac disease or cardiac events during follow-up is low. This does not apply to families with obvious familial SUDS.
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页码:1728 / 1732
页数:5
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